The Iran Thalassemia Prevention Program: Success or Failure?

BACKGROUND
Iran is one of the countries located on the "thalassemia belt" and a thalassemia prevention program was approved in our country in 1995. Many different researchers have studied the success of this program with no unanimous findings.


MATERIAL AND METHODS
A comprehensive literature search was performed using PubMed, Web of Science, and Google Scholar databases in Farsi and English languages for relevant articles published up to March 2015.


RESULTS
A total of 46 articles regarding thalassemia prevention were identified. After screening the titles and abstracts, 27 articles were excluded because they were the same articles, review articles, and case reports. Finally, 16 articles about the success of the Iranian thalassemia prevention program were selected for the evaluation.


CONCLUSION
The findings show that the program has been significantly successful in the reduction of the new thalassemia births, though not in a few provinces like Sistan and Baluchestan. The role of the network of genetic labs has been also indispensable in the reduction of the new births. However, there is ambiguity over the impact of the program on the attitude and awareness of people across the country about the prevention of inherited diseases. However, with the success of the Iran thalassemia prevention program, it needs to be modified to be more compatible with the relevant social textures of different provinces.


Results
A total of 46 articles regarding thalassemia prevention were identified. After screening the titles and abstracts, 27 articles were excluded because they were the same articles, review articles, and case reports. Finally, 16 articles about the success of the Iranian thalassemia prevention program were selected for the evaluation.

Conclusion
The findings show that the program has been significantly successful in the reduction of the new thalassemia births, though not in a few provinces like Sistan and Baluchestan. The role of the network of genetic labs has been also indispensable in the reduction of the new births. However, there is ambiguity over the impact of the program on the attitude and awareness of people across the country about the prevention of inherited diseases. However, with the success of the Iran thalassemia prevention program, it needs to be modified to be more compatible with the relevant social textures of different provinces.

Introduction
Thalassemia major is an inherited disease most prevalent in the region called thalassemia belt (1). Iran is one of the countries located on the belt with an average thalassemia gene prevalence rate of 4% (2). Upon the establishment of Iran Thalassemia Association in 1989, the need was felt to make measures for the prevention of thalassemic newborns in Iran (2). As a result, the first country-wide thalassemia prevention program was formulated in 1995 and started to be implemented across Iran in 1997 (3). The first study about the outcomes of the program was conducted by Samavat et al. which showed significant success in decreasing the rate of thallasemic newborns (4). Subsequent studies showed the exaggerated success reported in the first study due to the delay in the registration process of newborn thalassemic patients in Iran Health System which had not been considered by the researchers (5) . The second effort to evaluate the thalassemia prevention program in Iran was made by Abolghasemi et al. who considered two different phases in their study: the first phase during which the screening of marriage candidates was the only strategy to reduce the rate of thalassemia and the second phase in which the abortion in special medical cases received religious approval which made the use of prenatal diagnosis (PND ) possible in prevention of thalassemia. They concluded that the former phase was not that successful and the latter phase on the contrary was success (3). However, no statistics have been reported in their study to show their definition of success. Hadipour et al. in another study evaluated the success rate of the thalassemia prevention program during 2001-2006 period indicating the downward trend in the number of thalassemia new births as a success, though with heterogeneity in a few provinces, where the program was faced with serious challenges (5). In yet another study conducted by Hadipour et al., the emphasis has been placed again on the heterogeneity of the program in different provinces, though they evaluated the program to be successful (6). Miri et al. also studied about the success rate of the program in comparison with the neighboring Muslim countries and a few European countries like Greece and Cyprus; their findings showed the program to be successful as a model for the prevention of blood-borne diseases in developing Muslim countries (2). But there is not yet any consensus on the success of the program in Iran and many other researchers like Ghotbi et al. hold the belief that the program has many obstacles in its way to fulfill its goals (7). In the present review, given the importance of thalassemia prevention in the countries being located on the thalassemia belt, and considering the importance of the Iranian prevention program as a reliable model for developing Muslim countries, we have reviewed the success of the program considering its various aspects including the change it has made in the attitude of the society, the decrease in the number of thalassemia new births, and the effectiveness of the network of PND laboratories in Iran.

Search Strategy
A comprehensive literature search was performed using PubMed and Google scholar database for relevant articles published with the following key words "thalasemia,'' ''Iran,'' "Hemolytic disorder'', "Beta globin deficiency''. All eligible studies were published before March 30, 2014. In addition, studies were identified by a manual search of the reference lists of reviews and retrieved studies. We included all the case-control studies and cohort studies that investigated the thalassemia prevention program. All eligible studies were retrieved and their bibliographies were checked for other relevant publications. When the same sample was used in several publications, only the most complete study was included following careful examination.

Inclusion criteria
All studies were included if they met the following criteria: (1) cohort and case-control studies were considered, (2) evaluated the thalassemia prevention program. Major reasons for exclusion of studies were as follows: (1) not for thalassemia research and (2) duplicate of previous publication. Figure 1 graphically illustrates the trial flow chart. A total of 46 articles regarding thalassemia prevention were identified. After screening the titles and abstracts, 27 articles were excluded because they were the same articles, review articles, and case reports. Finally, 19 articles had addressed the success rate of the program research-wise both country-wide and local or small-scale. Many other research efforts in this regard had been made through theses and reported in non-ISI and non-PubMed Farsi or English journals; thus, further search by Google gave us 8 more relevant articles. The publ ished researches about the success of the program were categorized triple: those focusing on the decrease in the number of thalassemia new births, those addressing the change in the attitude of the society towards thalassemia prevention, and those evaluating the success rate of the network of PND laboratories in Iran. The findings of five of the articles were eligible to be included in more than one of the three categories above.   (5,6). Haddow displays the Iran thalassemia prevention program as an appropriate model for disease prevention and control that can be used as a successful program (13). Moradi et al. acknowledge the success of the program in the screening phase but recommend some changes (14). The other aspects of the program including its success in the attitude and behavior change of citizens should be also taken into account.  (3,6). Thus, more PND efforts should be exerted to elicit more success from the program.

Conclusions
All studies indicate the success of the thalassemia prevention program in Iran though with no consistency in all the provinces. Therefore, the program strongly requires to be made more native and compatible with the norms of the provinces especially in Sistan & Baluchestan where we are witness to many new thalassemia cases on an annual basis. Regarding the PND lab network, all findings unanimously show the increasing efficacy in the prevention of thalassemia; however, all the researchers recommend that the decision makers be more persistent for further and easier access of the carrier couples to PND. As far as the attitude and behavior changes are concerned, the program has been of little avail in the provinces different in their social context and norms. More importantly, except for the medical practitioners directly involved in the program, other medical specialty groups should be more informed on the program and the importance of thalassemia prevention. Since the Iran thalassemia prevention program is represented as a distinct model among the developing and Muslim countries, the success of the program can promote the value of the thalassemia prevention in the attributed countries located on the thalassemia belt. To increase the success, it is imperative to make the program more compatible with the native parameters of the provinces and perform regular evaluations over the program. Making efforts to clarify the importance of the thalassemia prevention for the young girls and boys before the marriage age and for all medical specialties can lead to stronger public incentive for voluntary participation in thalassemia prevention.